Gestação em Portadora de Hipercolesterolemia Familiar e Doença Aterosclerótica Prematura

Familiar xanthomatosis hypercholesterolemia in homozigous or heterozygous (two or more defects in LDL receptors) forms are rare. The cholesterol levels are frequently above 700 mg/dL. The management of these patients includes odd procedures, such as ileal bypass to control of levels of cholesterol. We present a case of pregnancy in patient with familiar hypercholesterolemia (FH) and coronary and cerebral atherosclerosis who had undergone ileal bypass, five years prior. During pregnancy, there were no clinical signs or symptoms related to coronary or cerebral atherosclerosis and we did not observe obstetric complications. Nevertheless, the levels of cholesterol and triglyceride increased significantly to 1182mg/ dL and 807mg/dL. Face the unknown prognosis of this clinical situation we decided to hospitalize the patient and to prescribe prolonged rest, dietary measures and specific therapy. This approach permited her to reach the end of pregnancy without maternal and fetal complications. The patient was submitted to cesarean section by obstetric reasons. The newborn was healthy but his levels of cholesterol and triglycerides were respectively, 339mg/ dL and 301mg/dL. The success of this case does not allow the change in the recommendation of avoiding pregnancy in patients with severe FH. Hipercolesterolemia familiar xantomatosa na forma homozigotica ou heterozigotica grave (HF) e rara. Os niveis sanguineos de colesterol frequentemente superiores a 700mg/dL exigem procedimentos terapeuticos invasivos nao habituais. Apresentamos caso de gestacao bem sucedida em portadora de HF associada a doenca aterosclerotica coronaria e cerebral, submetida a cirurgia de anastomose ileal parcial ha cinco anos para reducao dos niveis de lipideos. Durante a gestacao os niveis de colesterol e triglicerides atingiram 1182mg/dL e 807mg/dL, respectivamente, porem nao acompanhados de manifestacoes clinicas, sinais de progressao das lesoes arteriais obstrutivas ou intercorrencias obstetricas. Em funcao da evolucao clinica de HF grave na gestacao ser desconhecida e perante o mau prognostico, medidas higieno-dieteticas, repouso prolongado e terapeutica especifica foram adotadas, permitindo o alcance do termo da gestacao, sem complicacoes materno-fetais. A paciente deu a luz a recem-nascido vivo com niveis de colesterol total 339mg/dL e triglicerides de 301mg/ dL. O sucesso deste caso nao deve invalidar o atual desaconselhamento a gestacao em portadora de HF grave.